Abstract
We studied 7 girls, ages 1.3 to 10 yrs., with precocious puberty associated with MAS. In 6 of the 7 girls, basal and LHRH-stimulated gonadotropins and bioactive LH were prepubertal. In one girl, gonadotropins and bioactive LH were in the pubertal range. Ovarian volumes (mean 6.4, range 1.4 - 12.9 ml) were increased in all 7 girls compared to prepubertal girls (nl < 0.9 ml - Radiology 125:477(1977)). The girls with MAS had greater asymmetry in ovarian volume than did girls with true precocious puberty (TPP) (R to L difference 5.9 ± 1.9 ml vs. 1.5 ± 0.2 ml in TPP; p<0.05). The logarithm of plasma estradiol correlated significantly with ovarian volume (p< 0.05). Ovarian volumes and plasma estradiol fluctuated markedly over time. LHRH-analog treatment, given to 6 of the 7 girls, failed to suppress menses, ovarian cyst volume, or estradiol levels in the five girls who had low gonadotropins. The girl who had true puberty responded to LHRH-analog treatment. She had a bone age of 13.5 years, which is within the range when puberty normally occurs. Thus, we cannot exclude the possibility that her gonadotropins were also low when secondary sex characteristics first appeared. We conclude that the mechanism of precocious puberty in the majority of girls with MAS is intermittent ovarian estrogen secretion independent of pubertal hypothalamic-pituitary activation.
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Foster, C., Pescovitz, O., Shawker, T. et al. MECHANISM OF PRECOCIOUS PUBERTY IN GIRLS WITH McCUNE-ALBRIGHT SYNDROME (MAS). Pediatr Res 18 (Suppl 4), 167 (1984). https://doi.org/10.1203/00006450-198404001-00445
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DOI: https://doi.org/10.1203/00006450-198404001-00445